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C9ORF72-derived poly-GA DPRs undergo endocytic uptake in iAstrocytes and spread to motor neurons

Paolo M Marchi, Lara Marrone, Laurent Brasseur, Audrey Coens, Christopher P Webster, Luc Bousset, Marco Destro, Emma F Smith, Christa G Walther, Victor Alfred, Raffaele Marroccella, Emily J Graves, Darren Robinson, Allan C Shaw, Lai Mei Wan, Andrew J Grierson, Stephen J Ebbens, Kurt J De Vos, Guillaume M Hautbergue, Laura Ferraiuolo, Ronald Melki  Correspondence email, Mimoun Azzouz  Correspondence email
Published 13 May 2022. DOI: 10.26508/lsa.202101276

Article Information

vol. 5 no. 9 e202101276
DOI 
https://doi.org/10.26508/lsa.202101276
PubMed 
35568435
Published By 
Life Science Alliance
Online ISSN 
2575-1077
History 
  • Received October 26, 2021
  • Revision received May 3, 2022
  • Accepted May 4, 2022
  • Published online May 13, 2022.
Copyright & Usage 
© 2022 Marchi et al. This article is available under a Creative Commons License (Attribution 4.0 International, as described at https://creativecommons.org/licenses/by/4.0/).

Author Information

  1. Paolo M Marchi, Conceptualization, Data curation, Formal analysis, Validation, Investigation, Methodology, Writing—original draft1,3,
  2. Lara Marrone, Validation, Investigation, Methodology, Writing—original draft, review, and editing1,3,
  3. Laurent Brasseur, Resources, Methodology2,
  4. Audrey Coens, Methodology2,
  5. Christopher P Webster, Resources, Methodology, Writing—review and editing1,3,
  6. Luc Bousset, Resources, Methodology, Writing—review and editing2,
  7. Marco Destro, Resources1,3,
  8. Emma F Smith, Resources, Methodology1,3,4,
  9. Christa G Walther, Software, Validation, Methodology5,
  10. Victor Alfred, Resources, Methodology1,3,
  11. Raffaele Marroccella, Resources, Methodology1,3,
  12. Emily J Graves, Resources, Data curation, Formal analysis, Methodology1,3,
  13. Darren Robinson, Resources, Data curation, Formal analysis, Methodology5,
  14. Allan C Shaw, Resources, Methodology1,3,
  15. Lai Mei Wan, Resources, Methodology1,3,
  16. Andrew J Grierson, Resources1,3,
  17. Stephen J Ebbens, Resources, Software, Methodology6,
  18. Kurt J De Vos, Resources, Methodology1,3,4,
  19. Guillaume M Hautbergue, Conceptualization, Supervision, Writing—review and editing1,3,
  20. Laura Ferraiuolo, Resources, Supervision, Investigation, Methodology, Writing—review and editing1,3,
  21. Ronald Melki, Resources, Methodology, Writing—review and editing2⇑ and
  22. Mimoun Azzouz, Conceptualization, Supervision, Funding acquisition, Project administration, Writing—review and editing1,3⇑
  1. 1Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, UK
  2. 2The French Alternative Energies and Atomic Energy Commission (CEA), Institut François Jacob (MIRcen) and The French National Centre for Scientific Research (CNRS), Laboratory of Neurodegenerative Diseases (UMR9199), Fontenay-aux-Roses, France
  3. 3Neuroscience Institute, University of Sheffield, Western Bank, Sheffield, UK
  4. 4Centre for Membrane Interactions and Dynamics, University of Sheffield, Western Bank, Sheffield, UK
  5. 5The Wolfson Light Microscopy Facility, University of Sheffield, Sheffield, UK
  6. 6Department of Chemical and Biological Engineering, University of Sheffield, Sheffield, UK
  1. Correspondence: ronald.melki{at}cnrs.fr; m.azzouz{at}sheffield.ac.uk
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Funding

  • European Research Council

    294745
    M Azzouz

  • MRC DPFS Award

    129016
    M Azzouz

  • JPND-MRC

    MR/V000470/1
    M Azzouz

  • ARUK award

    ARUK-PG2018B-005
    M Azzouz

  • CureAP4

    M Azzouz

  • Maddi Foundation

    L Marrone, M Azzouz

  • Spastic Paraplegia Foundation

    L Marrone, M Azzouz

  • British Neuropathological Society

    L Marrone

  • Motor Neurone Disease Association Prize Studentship

    DeVos/Oct13/870-892
    EF Smith, KJ De Vos, AJ Grierson

  • Motor Neurone Disease Association project grant

    DEVOS/APR18/862-79
    EF Smith, KJ De Vos, AJ Grierson

  • Medical Research Council

    MR/S025979/1, MR/M013251/1, MR/R024162/1
    KJ De Vos, GM Hautbergue, L Ferraiuolo

  • European Commission Joint Programme on Neurodegenerative Diseases

    JPND-TransPathND, ANR-17-JPCD-0002-02
    L Brasseur, L Bousset, R Marroccella

  • Electron microscopy facility of Imagerie-Gif

    ANR-10-INBS-04-01, ANR-11-IDEX-0003-02

  • Biotechnology and Biological Sciences Research Council

    BB/S005277/1
    GM Hautbergue

  • Academy of Medical Sciences

    SBF002\1142
    L Ferraiuolo

  • MRC

    1812144
    L Ferraiuolo
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C9ORF72 DPRs spread from iAstrocytes to motor neurons
Paolo M Marchi, Lara Marrone, Laurent Brasseur, Audrey Coens, Christopher P Webster, Luc Bousset, Marco Destro, Emma F Smith, Christa G Walther, Victor Alfred, Raffaele Marroccella, Emily J Graves, Darren Robinson, Allan C Shaw, Lai Mei Wan, Andrew J Grierson, Stephen J Ebbens, Kurt J De Vos, Guillaume M Hautbergue, Laura Ferraiuolo, Ronald Melki, Mimoun Azzouz
Life Science Alliance May 2022, 5 (9) e202101276; DOI: 10.26508/lsa.202101276
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Volume 5, No. 9
September 2022
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